Endocrine Abstracts

Case history: A 74 years old gentleman with no medical illness presented with loss of consciousness in March 2018. CT head did not show acute cortical infarct but there was florid symmetrical calcification involving the basal ganglia, thalami, cerebellar lobes and subcortical white matter within the parietal-occipital lobes bilaterally. Fahr’s disease was suspected and an outpatient MRI was requested.

Investigation and Results: 2 weeks later, a routine blood investigation with patient’s GP note a corrected calcium level of 1.39 mmol/l. Patient was asymptomatic and was admitted for calcium replacement. Vitamin D level was 43, albumin 44, phosphate 1.31, magnesium 0.92 and parathyroid hormone 0.4. In-patient MRI showed bilateral basal ganglia and subcortical white matter calcification, consistent with the findings from the previous CT.

Diagnosis and Treatment: Diagnosis of primary hypoparathyroidism was made. Brain calcification on CT and MRI is most likely due to hypoparathyroidism. Calcium tablets and alfacalcidol was started and the patient’s corrected calcium level gradually improved and normalised. The patient was also referred to the cardiology team because of trifascicular heart block on ECG. 24 hours tape showed runs of VT. Diagnosis of tachy-brady syndrome was made and he will likely need a pacemaker if a repeated 24 tape after correction of his calcium level still show arrhythmias. He is also seeing ophthalmology and awaiting cataract surgery. The patient was last seen in the clinic a week ago and his calcium level is in the normal range with calcium and alfacalcidol supplement. His ECG was normal with no heart block. USG KUB did not show nephrocalcinosis.

Conclusion and points for discussion: Hypoparathyroidism must be suspected in a patient with basal ganglia calcifications and calcium level should be checked.

1. There are several features that are unique to chronic hypoparathyroidism. These include the presence of basal ganglia calcifications, cataracts, dental abnormalities, and ectodermal manifestations.

2. Acquired hypoparathyroidism is most often the result of post-surgical or autoimmune damage to the parathyroid glands.

3. Persistent hypocalcemia with a low or inappropriately normal parathyroid hormone level and hyperphosphatemia is, in the absence of hypomagnesemia, virtually diagnostic of hypoparathyroidism.

4. The goals of therapy in patients with hypoparathyroidism are to relieve symptoms, to raise and maintain the serum calcium concentration in the low normal range ((2.0 to 2.1 mmol/l)), and to prevent iatrogenic development of kidney stones.